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Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry

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Background and Aims Monitoring Wilson disease (WD) is challenging due to its variable presentation and the absence of reliable biomarkers. This study aims to assess the predictive value of liver enzymes, particularly transaminases, on long-term outcomes in patients with hepatic WD using data from the Spanish Wilson Registry. Patients and Methods We analysed data from 162 WD patients with hepatic involvement and over one year of follow-up. Patients were classified as mild (no cirrhosis) or severe (with cirrhosis) at diagnosis. An unstable pattern of transaminases was defined as recurrent AST or ALT elevations. Unfavourable outcomes included new cirrhosis, elastography progression > 2 Kpa, liver transplant, or liver-related deaths. Logistic regression models were used to evaluate the impact of various factors on disease outcome. Results Of 162 patients, 81.5% had mild disease at diagnosis. Most received chelators as first-line therapy, achieving an 81.4% one-year biochemical response. After a median follow-up of 17 years, 59% exhibited an unstable transaminase pattern, and 29% had an unfavourable outcome. Key factors associated with poor outcome included older age at diagnosis (OR = 1.03), lack of early biochemical response (OR = 0.19), advanced disease markers (platelet count, albumin), and an unstable transaminase pattern (OR = 2.92). Transaminase levels did not predict outcomes based on initial disease severity. Even patients with mild disease at diagnosis and persistently normal transaminases could experience progression over time, underscoring the need for more thorough follow-up evaluations. Conclusion While transaminases are valuable for monitoring WD, they should be used alongside other biomarkers to better predict disease progression.

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Orphanet Journal of Rare Diseases, 2025, vol. 20, num. 1

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Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))

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Predictive value of liver enzymes in long-term prognosis of hepatic Wilson disease: results from the Wilson AEEH registry